Congenital adrenal hyperplasia (CAH) is a rare autosomal recessive disorder with mutations in genes involved in cortisol and aldosterone production. Based on overall 21-OHase activity, CAH is divided into classic (C-CAH) and non-classic (NC-CAH). Females who suffered from NC-CAH have had increased infertility rates and higher miscarriage susceptibility. The treatment of CAH in pregnancy is still debatable.

We present 22 years old pregnant female (seventh week of gestation), who is currently under dexamethasone (DEX) since almost seven years for NC-CAH. At presentation, she is normotensive, non-obese, with no signs of hirsutism and Cushing syndrome. Seven days after the first visit, an endocrinologist makes informative talk with the patient and her mother about NC-CAH, pregnancy, and drugs-associated risks. Current Clinical Practice Guideline for CAH treatment suggests the use of protocols approved by Institutional Review Boards at Centers experienced in CAH treatment.

In women with CAH who are planning a pregnancy, a close relationship between endocrinologist, reproductive gynaecologist and molecular biologist is of great interest. Prenatal management with DEX is advised in particular circumstances. In remaining, the switch from DEX to other glucocorticoids that do not penetrate placenta is advised.